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Introduction: Over the last decades, mini-invasive surgery has become increasingly common for treatment of primary hyperparathyroidism; such approach requires preoperative localization of a suspected parathyroid adenoma. Neck ultrasound (US) and technetium-99 m sestamibi (MIBI) scan are the main imaging studies used for this purpose. The aim of the present study is to evaluate what pre- and post-operative factors may alter the reliability of localization studies.Methods: A retrospective analysis on 212 patients with preoperative diagnosis of primary hyperparathyroidism was conducted. Data collected included demographic data, preoperative workup, operative findings and follow-up. Univariate logistic regression was performed on pre- and postoperative variables.Results: US sensitivity was 62.4% and MIBI sensitivity 78.9%. Cure rate after parathyroidectomy was 98.1%. Univariate logistic regression demonstrated that US sensitivity was impaired by lower levels of serum calcium (p < 0.0001), multi-gland disease (p = 0.011) and co-existence of thyroid disease (p = 0.001); MIBI sensitivity was impaired by lower levels of serum calcium (p = 0.001) and multi-gland disease (p < 0,0001).Conclusions: Mild hypercalcaemia, multi-gland disease and co-existing thyroid disease are the main factors affecting sensitivity of preoperative imaging studies. In such patients a mini-invasive approach is possible but the use of intraoperative PTH monitoring is mandatory to reduce the risk of unsuccessful surgery. [ABSTRACT FROM AUTHOR]

Background: Parathyroid hormone (PTH) monitoring during the surgical procedure can confirm the removal of all hyperfunctioning parathyroid tissue, as the half-life of PTH is approximately 5 min. The commonly applied Irvin criterion is reported to correctly predict post-operative calcium levels in 96-98% of patients. However, the PTH baseline reference concentration is markedly influenced by surgical manipulations during preparation of the affected glands, interindividual variability of the PTH half-life and modifications in the physiological state of the patient during surgery. The aim of this study was to evaluate the possible impact of the measurement of intraoperative PTH 20 minutes after surgery. Methods: Between 2003 and 2012, 188 patients underwent a focused parathyroidectomy associated to rapid intraoperative PTH assay monitoring. Blood samples were collected: 1) at pre-incision time, 2) at 10 min after gland excision and 3) at 20 min after excision, if a sufficient reduction of PTH value was not observed. On the bases of the Irvin criterion, an intra-operative PTH drop>50% from the highest either pre-incision or pre-excision level after parathyroid excision was considered a surgical success. Results: A >50% decrease of PTH after gland excision compared to the highest pre-excision value occurred in 156/188 patients (83%) within 10 min and in further 12/188 after 20 minutes (6.4%). In the remaining 20 patients (10.6%) values of PTH remained substantially unchanged or decreased less than 50% and for this reason bilateral neck exploration was performed. An additional pathologic parathyroid was removed in 9 cases, a third in one. In the other 10 cases further neck exploration by a standard cervical approach was negative and in four of these persistent postoperative hypercalcemia was demonstrated. The overall operative success was 97.3%. Intraoperative PTH monitoring was accurate in predicting operative success or failure in 96.3% of patients. Conclusions: The 20 minutes PTH measurement appears very useful, avoiding unnecessary bilateral exploration and the related risk of complications with only a slight increase of the duration of surgery and of the costs. PTH values decreasing appeared to be influenced by surgical manipulations during minimally invasive parathyroidectomy. [ABSTRACT FROM AUTHOR]

Design: To evaluate the efficacy of the main tools in the diagnostic localization of hyperfunctioning parathyroids (HP) in primary hyperparathyroidism (pHPT) with concomitant thyroid diseases. Methods: Forty-three patients with pHPT associated with nodular goiter (NG, n=32) and/or autoimmune thyroid diseases (AITDs, n=11) for a total of 63 neck lesions were considered. Sixteen patients displaying HP (16 lesions), unequivocally localized by sestaMIBI scintigraphy (MIBI) and neck ultrasound (US) (group I), were compared with 27 patients (47 neck lesions) displaying equivocal parathyroid localization (group II). In all cases, neck US, MIBI scan, cytology, and parathyroid hormone assay in fine-needle aspiration washout fluid (FNA-PTH) were performed. All patients finally underwent surgery. Results: According to histological examination, high FNA-PTH values (>103 pg/ml) correctly identified all HP in both groups of patients (100% of sensitivity and specificity). Both MIBI and US correctly identified all HP only in group I patients; in contrast, four patterns of mismatch between these techniques were observed in group II patients, leading to low diagnostic performances of neck US (71.4% sensitivity and 78.9% specificity) and of MIBI scan (35.7% sensitivity and 42.1% specificity). The latter was due to both false-negative (mainly in AITD) and false-positive (mainly in NG) scan images. Conclusions: Coexistent thyroid diseases are responsible for mismatch between MIBI and US images resulting in equivocal HP localization. In these cases, FNA-PTH resulted in the most accurate tool to identify HP. However, although safe, it should be advised only to patients with uncertain HP localization. [ABSTRACT FROM AUTHOR]

Background: Diagnosis of multiple endocrine neoplasia type 1 (MEN1) is commonly based on clinical criteria, and confirmed by genetic testing. In patients without known MEN1-related germline mutations, the possibility of a casual association between two or more endocrine tumors cannot be excluded and subsequent management maybe difficult to plan. We describe a very uncommon case of functioning glucagonoma associated with primaryhyperparathyroidism (pHPT) in which genetic testing failed to detect germline mutations of MEN-1 and other known genes responsible for MEN1.Case presentation: The patient, a 65-year old woman, had been suffering for more than 1 year from weakness,progressive weight loss, angular cheilitis, glossitis and, more recently, skin rashes on the perineum, perioral skin and groin folds. After multidisciplinary investigations, functioning glucagonoma and asymptomatic pHPT were diagnosed and, since family history was negative, sporadic MEN1 was suspected. However, genetic testing revealed neither MEN-1 nor other gene mutations responsible for rarer cases of MEN1 (CDKN1B/p27 and other cyclin-dependent kinase inhibitor genes CDKN1A/p15, CDKN2C/p18, CDKN2B/p21). The patient underwent distalsplenopancreatectomy and at the 4-month follow-up she showed complete remission of symptoms. Six months later, a thyroid nodule, suspected to be a malignant neoplasia, and two hyperfunctioning parathyroid glands were detected respectively by ultrasound with fine needle aspiration cytology and 99mTc-sestamibi scan with SPECT acquisition. Total thyroidectomy was performed, whereas selective parathyroidectomy was preferred to a more extensive procedure because the diagnosis of MEN1 was not supported by genetic analysis and intraoperative intact parathyroid hormone had revealed “adenoma-like” kinetics after the second parathyroid resection. Thirty-nine and 25 months after respectively the first and the second operation, the patient is well and shows no signs or symptoms of recurrence.Conclusions: Despite well-defined diagnostic criteria and guidelines, diagnosis of MEN1 can still be challenging.When diagnosis is doubtful, appropriate management may be difficult to establish. [ABSTRACT FROM AUTHOR]

Parathyroid carcinoma is a rare malignancy representing less than 1% of primary hyperparathyroidism cases. Its management is controversial due to lack of large-scale, multicentric studies. We report 8 new cases of parathyroid carcinoma and review the literature. Preoperative diagnosis of carcinoma was possible in 2 (25%) cases. Unclear surgical margins were present in 5 (62.5%) patients; 4 of them underwent subsequent re-exploration and ipsilateral hemithyroidectomy, in one case associated to central lymph node dissection. Recurrent disease is reported in 2 (25%) patients. Considering the high incidence of local recurrence in case of unclear surgical margins, a re-exploration with ipsilateral hemithyroidectomy is indicated in these patients. A neck dissection should be performed only in case of clinically involved lymph nodes, avoiding prophylactic lymphectomy. An aggressive approach is indicated in case of local or distant recurrence to reduce hypercalcemia. [ABSTRACT FROM AUTHOR]